What Is Sydenham Chorea?
Sydenham chorea is a neurological condition prevalent in children due to infection from group-A beta-hemolytic streptococcus. This is the same bacteria that causes rheumatic fever. It is a rare disorder characterized by the sporadic onset of chorea. Chorea is defined as abnormal and involuntary movement of the body parts. Chorea may be manifested in arms, legs, tongue, face, etc. After contracting group A beta-hemolytic streptococcal infection, Sydenham chorea typically develops within a few weeks to months. It can appear as an isolated finding or a serious consequence of acute rheumatic fever. Sydenham disorder is an autoimmune condition (a condition in which the body’s immune system starts attacking its healthy cells). These bacteria can react with basal ganglia (a part of the brain) to cause Sydenham chorea. The basal ganglia help to control movement, posture, speech, etc.
What Are the Other Names of Sydenham Chorea?
Alternate names of Sydenham chorea are:
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Rheumatic chorea.
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Saint Vitus dance.
What Causes Sydenham Chorea?
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Sydenham chorea is an autoimmune condition (a condition in which the immune system starts attacking healthy cells of the body).
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Most instances occur after a streptococcal infection or a more serious rheumatic fever.
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Streptococcal infection in Sydenham chorea causes the body's immune system to produce antibodies to fight the infection. Unfortunately, these antibodies continue to exist for unknown reasons and subsequently attack certain cells in the joints, kidneys, heart, and brain, particularly basal ganglia. These basal ganglia are crucial for controlling the body's movements; therefore, it causes characteristic symptoms of Sydenham chorea.
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More information is needed regarding the precise underlying mechanisms that lead to chorea. The antigens on streptococcal bacterial cells are analogous to human body cells in their ability to stimulate an immune response. In genetically predisposed individuals, antibodies produced by the immune system to fight the streptococcal infection may erroneously bind to healthy cells. This may lead to disturbance in the signaling and movement controlling the brain cells.
What Are the Symptoms of Sydenham Chorea?
Symptoms of Sydenham chorea include the following:
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After a streptococcal infection, Sydenham chorea symptoms might occur anywhere between one week and six months.
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Involuntary and short movements of the body which look like restlessness or dancing movements. Sydenham chorea is characterized by abnormal movements (chorea) that often appear over hours and culminate within a few hours or days.
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Clumsy movements.
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Difficulty in maintaining the grip of hands.
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The unusual movements associated with Sydenham chorea can range from mild symptoms that impair coordination and simple tasks like writing to severe symptoms that interfere with walking, talking, dressing, eating, or even just holding things. These motions may change during the day. However, most of the time, the chorea goes away while sleeping.
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People with Sydenham chorea may experience a sudden and quick movement of muscles, obsessiveness, compulsions, lack of attention, anxiety, exaggerated changes in mood, reduced verbal output, etc.
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Moreover, the patient may experience extreme muscle weakness and dysarthria (slurred speech).
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In less than two percent of the cases, muscle weakness, easy irritability, confusion, and altered mental state may be common, and the affected child may become bedridden. This condition is called paralytic chorea.
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Because Sydenham chorea is a complication of rheumatic fever, some patients also experience joint arthralgia (pain in joints) or arthritis (swelling in joints), heart valve inflammation that results in long-term damage, and persistent fever.
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Symptoms of Sydenham chorea typically go away in three to six months. However, the duration of the symptoms could exceed a year. Sometimes Sydenham chorea symptoms can resurface in adulthood, especially in women during pregnancy (first trimester).
Is Sydenham Chorea a Common Condition?
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Sydenham chorea is prevalent in children aged 5 to 15 years.
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Sydenham chorea more commonly affects girls than boys.
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Sydenham chorea has rarely been reported in children under five or adults.
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This condition affects people of all races and ethnicities equally.
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Rheumatic fever can lead to Sydenham chorea. About 25 percent of people who have rheumatic fever develop Sydenham chorea.
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The most common cause of acute chorea in children in the United States is Sydenham chorea.
How Is Sydenham Chorea Diagnosed?
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A thorough clinical evaluation, complete patient history, and detection of newly appearing choreic movements are used to diagnose Sydenham chorea.
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A history of streptococcal infection or detection of infection through throat swab tests or the presence of streptococcal antibodies in the blood is also helpful in confirming the diagnosis of Sydenham chorea.
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Moreover, comorbidities like pain or swelling in joints or cardiac valve problems also help confirm the diagnosis.
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Specific imaging procedures like magnetic resonance imaging (MRI) may occasionally be advised to rule out other causes. Brain MRI in Sydenham chorea is frequently done.
How Is Sydenham Chorea Treated?
The child's symptoms will determine which treatments are most effective. Treatment might not be necessary in mild cases of Sydenham chorea. The treatment modalities involve the following:
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Secondary Prevention: This therapy aims to stop the child's risk of developing permanent heart valve damage from repeated streptococcal infections. Antibiotics like penicillin may be used for a long time (maybe until adulthood). In addition, doctors should follow the latest rheumatic fever guidelines.
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Chorea-Suppressing Treatment: Low dosages of powerful dopamine receptor blockers (Haloperidol), dopamine-depleting drugs (Tetrabenazine), anti-seizure drugs (Valproic acid), or Benzodiazepines may be helpful when chorea symptoms are severe and interfere with patient's ability to function normally.
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Immune System Therapy: Immune treatments are useful in treating individuals with severe Sydenham chorea in the first weeks of the onset of symptoms. Based on the hypothesis that persistent acute inflammation contributes to the symptoms. The use of oral steroids and intravenous immunoglobulins helps in treating the symptoms.
Can Sydenham Chorea Be Prevented?
The incidence of this condition may be decreased by prompt strep throat and rheumatic fever treatment. Moreover, being on antibiotics until adulthood can reduce the chance of recurrent streptococcal infections and the risk of heart disease in children who have experienced Sydenham chorea.
Conclusion
Sydenham chorea is a rare autoimmune condition that often occurs after a streptococcal infection. Most children recover completely in three to six months. However, only a small percentage of children experience symptoms for up to two years. In some cases, symptoms can recur later in life, particularly in those who use Estrogen, take birth control pills, or are in the first trimester of pregnancy.
